Is Narcolepsy a Neurodegenerative Disorder?

Human narcolepsy is a complex disorder characterized by a tetrad of symptoms: excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis. Patients rarely have all four symptoms, and two main types of narcolepsy have been descr

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Christian R. Baumann Claudio L. Bassetti Thomas E. Scammell Editors

Narcolepsy Pathophysiology, Diagnosis, and Treatment

Editors Christian R. Baumann, MD Department of Neurology University Hospital of Zurich Zurich, Switzerland [email protected] Claudio L. Bassetti, MD Department of Neurology Neurocenter EOC of Southern Switzerland Ospedale Civico Lugano, Switzerland [email protected]

Thomas E. Scammell, MD Department of Neurology Beth Israel Deaconess Medical Center and Harvard Medical School Boston, MA, USA [email protected]

ISBN 978-1-4419-8389-3     e-ISBN 978-1-4419-8390-9 DOI 10.1007/978-1-4419-8390-9 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2011926991 © Springer Science+Business Media, LLC 2011 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, ­electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com)

Preface

Narcolepsy is characterized by excessive daytime sleepiness, cataplexy, fragmented sleep, and other symptoms. It affects approximately 1 in 2,000 people and can have a huge impact on their ability to succeed in school and work. Narcolepsy was first recognized by clinicians over 125 years ago, yet until recently, its cause remained a mystery. In 2000, two research groups discovered that narcolepsy is caused by a selective loss of neurons in the hypothalamus that produce the hypocretin neuropeptides (also known as orexins). With this groundbreaking perspective, narcolepsy research has advanced in large steps, with new discoveries every year that have enhanced our understanding of the disorder. In 1975, the First International Symposium on Narcolepsy was held in La Grande Motte in France, organized by William C. Dement, Christian Guilleminault, and Pierre Passouant. After a successful Fifth International Symposium on Monte Verità near Ascona (Switzerland) in 2004, many of the world’s leading narcolepsy researchers – including the authors of this book – gathered again in this inspiring la